We read with interest the paper by Crosignani and colleagues (1) demonstrating the inefficacy of ursodeoxycholic acid (UDCA) in preventing cholestatic episodes in a patient with benign recurrent intrahepatic cholestasis (BRIC). We would like to report a different experience with a 24-yr-old woman affected by BRIC and observed in our pediatric clinic since she was 7 yr old. The detailed clinical history has been reported previously (2). Briefly, she had, since 1970, seven attacks of cholestasis (one during her only pregnancy). Each was preceded, in the 2 wk before the onset of jaundice, by typical prodromic symptoms such as anorexia, fatigue, weight loss, insomnia, abdominal pain and pruritus. The average duration of jaundice during the seven attacks was 16 wk, and the average peak total bilirubin 18.5 mg/dl. A liver biopsy specimen during a symptomatic period at age 8 showed marked centrolobular and intracellular cholestasis. At age 14, cholangiography was conducted and was reported to be normal. During the years from 1977 to 1987 early administration of high-dose cholestyramine and phenobarbital failed to reduce the duration of the episodes of cholestasis. On no occasion did a prodromal period fail to evolve into an episode of cholestatic jaundice.
In April 1988, she again experienced pruritus, anorexia, fatigue, marked weight loss and insomnia. Biochemical evidence of cholestasis was present (Table ). Treatment with 300 mg of UDCA twice a day was tentatively prescribed. Within 2 wk, all symptoms disappeared. After 2 mo, the dose of UDCA was tapered to 150 mg at bedtime and then gradually stopped. She was symptom-free during the successive 3 mo, then she again developed the usual prodromic features. She was again treated with UDCA, with a disappearance of symptoms and normalization of bilirubin values within 1 wk (Table ). During the last 4 yr, she has had no recurrence of cholestatic attacks. However, on two different occasions the occurrence of prodromic symptoms prompted us to prescribe UDCA successfully.
In this patient UDCA treatment prevented recurrence of cholestatic episodes over a 4-yr period of follow-up. Our experience is in contrast with the very well-detailed report of Crosignani et al. (1) suggesting different biochemical mechanisms of canalicular cholestasis in BRIC. Moreover, UDCA treatment was recently re-3118139348
TABLE. Laboratory data before (May 1988) and